Vital Importance of Delineation of Coronary Artery Anatomy in Atypical Congenital Giant Right Atrial Aneurysm

Giant congenital right atrial aneurysm is a very rare congenital heart lesion, which may be asymptomatic or present a variety of symptoms, particularly supraventricular arrhythmias and intracardiac thrombosis formation. This is a report on a 3.5-month-old male infant with imperforated anus and an unusual-shaped congenital giant right atrial aneurysm with retro-ventricular extension. This unusual shape prevented appearance of cardiomegaly on the chest X-Ray. Surgical resection of the aneurysm was attempted. However, posterior descending coronary artery, which was embedded in the wall of the aneurysm, was irreversibly damaged during the operation. The patient died in the operation room. We concluded that pre-operative delineation of coronary arteries in cases with congenital giant right atrial aneurysm (CGRAA) with extension to the posterior left ventricle is mandatory. Despite the current data that surgical excision of the aneurysm is the treatment of choice, our case required simple closure of the aneurysmal neck from inside the right atrium to be an easier and safer surgical approach for treatment of CGRAA with a tricky anatomy. This case indicates that delineation of coronary artery anatomy in atypical congenital giant right atrial aneurysm is of vital importance. Closure of the aneurysmal sac, instead of aneurysmal resection, is a safer and more simple approach in atypical cases.